Antigen receptor-mediated transmembrane signaling in Wiskott-Aldrich syndrome

J Immunol. 1994 Jul 1;153(1):395-9.

Abstract

The X-linked immunodeficiency Wiskott-Aldrich syndrome (WAS) is a condition that includes a deficient anti-polysaccharide Ab response. Recently, it has been suggested that B cells from patients with WAS show a defective calcium mobilization response upon engagement of sIgM. Because primarily EBV-transformed cells were used in these studies, we tested freshly isolated blood B cells for their calcium mobilization capability upon engagement of sIg and CD19. No significant differences in the calcium mobilization capability of CD20+ B cells of four individual WAS patients compared with capability in normal controls were found. Receptor desensitization as assessed by calcium mobilization inhibition also seemed to be intact. T cells were tested for their anti-CD3-induced calcium flux and, again, no abnormalities could be observed when compared with T cells from healthy individuals. We conclude that WAS B and T cells can be stimulated into a normal calcium mobilization response when their AgRs are cross-linked. It is highly improbable that the immune dysfunction observed in WAS patients is related to a direct disorder of their B and/or T cell AgRs.

MeSH terms

  • Adolescent
  • Adult
  • Antigens, CD / physiology
  • Antigens, CD19
  • Antigens, Differentiation, B-Lymphocyte / physiology
  • B-Lymphocytes / physiology*
  • Calcium / metabolism
  • Child
  • Humans
  • Lymphocyte Activation
  • Receptors, Antigen, B-Cell / physiology*
  • Receptors, Antigen, T-Cell / physiology*
  • Receptors, Complement 3d / analysis
  • Signal Transduction
  • T-Lymphocyte Subsets / physiology*
  • Wiskott-Aldrich Syndrome / immunology*

Substances

  • Antigens, CD
  • Antigens, CD19
  • Antigens, Differentiation, B-Lymphocyte
  • Receptors, Antigen, B-Cell
  • Receptors, Antigen, T-Cell
  • Receptors, Complement 3d
  • Calcium